Pleomorphic primitive neuroectodermal tumor with glial and neuronal differentiation: clinical, pathological,...

This paper examines a case of pleomorphic primitive neuroectodermal tumor (PNET) with glial and neuronal differentiation in a 7-year-old girl who was clinicopathologically reported with immunohistochemical and chromosomal analysis. Clinically, a highly aggressive behavior leading to early recurrence with cerebrospinal fluid seedings was characteristic despite total removal and chemoradiation therapy. Pathologically, severe pleomorphism was noted and large ganglioid cells were predominant. Immunohistochemically, the expression of low-molecular neurofilament was recognized in the surgical specimens and increased in the recurrence. Coexpression of vimentin and neurofilament/GFAP was recognized in the culture. Chromosomal analysis showed near-diploidy, but different karyotype from that PNETs previously reported. These findings suggested that PNETs with pleomorphism and differentiation into both glial and neuronal lineages may show aggressiveness and require more aggressive therapy.